TABLE 3.

Nevoid Basal Cell Carcinoma Syndrome Diagnostic Criteria

A diagnosis can be made if an individual meets 2 major criteria or 1 major + 2 minor criteria
Major criteria	Minor criteria
Basal cell carcinomas: ≥5 in a lifetime or 1 diagnosed before age 30	Childhood medulloblastoma
Lamellar calcification of the falx before the age of 20	Lympho-mesenteric or pleural cysts
Jaw odontogenic keratocyst	Macrocephaly (occipitofrontal circumference (OFC) > 97th percentile)
Palmar or plantar pits: ≥2	Cleft lip/palate
First-degree relative with NBCCS	Vertebral or rib anomalies (bifid/splayed/extra ribs, bifid vertebrae)
	Preaxial or postaxial polydactyly
	Ovarian or cardiac fibromas
	Ocular anomalies (cataract, developmental defects, pigmentary changes of the retinal epithelium)